Cranial nerve pathology, Dr. Nelson

• Bell’s Palsy
  • Most common cause of unilateral facial paralysis
  • Presentation
    • Acute unilateral facial paralysis with involvement of the forehead
  • Most common cause is idiopathic but there is association with HSV
  • Must exclude
    • Ear infection
    • Stroke
      • Forehead spared in central causes except if you have ipsilateral pontine pathology you can have forehead involvement and peripheral nerve presentation however will usually have CN VI involvement (check EOM)
    • Ramsay-Hunt syndrome from Herpes Zoster
    • Lyme disease (MCC bilateral Bell’s Palsy)
  • Tx
    • Steroids
      • Reduces relative risk of incomplete recovery at 6-12 mo
      • Prednisone 60-80 mg qd x 1 week
      • Ideal to start within 72 hours of Sx
    • Antivirals controversial
    • Supportive care if they cannot completely close their eye too keep eye moist and avoid corneal ulcers
  • Prognosis
    • 15% can have permanent involvement
    • Follow up with ENT in 1 week
• Trigeminal neuralgia
  • Paroxysms of severe unilateral pain lasting only seconds usually in the V2, V3 dermatome
  • 80-90% caused by compression from aberrant loop of artery/vein
    • Can also be 2/2 MS, malignancy, AVM
  • Tx
    • IV phenytoin/Fosphenytoin
      • Abortive Tx lasts 4 hr – 72 hr
    • Carbamazepine
      • First line outpatient Tx
      • High risk of side effects
    • Posterior fossa microvascular decompression surgery successful in 70% of patients

Temporal arteritis, Dr. Boland

• Temporal arteritis
  • Giant cell arteritis 
    • Granulomatous, medium to large vessel vasculitis
    • Females 3x more likely
    • Rule of 50s
      • 50 years of age, ESR > 50, treated with 50 mg prednisone daily
    • Cain cause painless ischemic optic neuropathy and blindness
  • Usually presents as a headache 85% of the time, can have jaw claudication, polymyalgia rheumatica seen in 50%, transient vision loss
  • Dx is confirmed by biopsy but if suspected start high dose corticosteroids prior to biopsy
    • If vision at any point during Hx loss admit, start IV steroids (methylpred), and have optho see
    • If no vision loss start high dose steroids (PO prednisone) and have optho see as soon as possible outpatient and biopsy between 1-2 weeks
  • ESR doesn’t have to be elevated (about 15% of time its not)

Pediatric endocrinology, Dr. Kopp

• DKA
  • Considerations regarding fluid administration and cerebral edema in peds
    • PECARN DKA Fluid Trial
      • Compared fast and slow infusions of normal and half normal saline (4 arms)
      • Afterwards performed bedside evaluation of neurologic status (this is a clinical Dx not radiologic)
        • Bimodal distribution for presentation of cerebral edema
          • 4 hours and 14 hours
      • 3.5% had GCS decline <14, 0.9% had clinically apparent brain injuries
      • *Conclusion: neither the rate of administration nor the sodium chloride content of the IVF had contribution to the neurologic outcomes
    • Fluid replacement calculations
      • Fast replacement
        • Assume 10% weight-based fluid deficit, give the 20 cc/kg bolus isotonic IVF and replace the remaining with 2x maintenance over 24h
        • Dr. Kopp’s opinion: 0.45 NaCl given as a fast replacement strategy is preferred method as there was a higher incidence of hyperchloremic metabolic acidosis in the normal saline group (not statistically significant but study was perhaps underpowered)
      • Slow replacement
        • Assume 5% deficit give the 10 cc/kg bolus isotonic IVF and replace the remaining with 1.5 x maintenance over 48h
    • Dextrose containing fluids to be added when glucose is 200-300 (i.e. ~250) or when there is > 100 drop in glucose between 1hr POC glucose checks
• Hypoglycemia
  • Rule of 50
    • Google and review it, V important
  • Consider inborn errors of metabolism in the differential of children who are hypoglycemic 
    • Children with inborn errors of metabolism who present with acute illness, nausea, vomiting need prompt evaluation and immediate initiation of IV dextrose containing fluids and give them oral glucose immediately while IV access is being established. They can decompensate rapidly if kept in a catabolic state
• Adrenal insufficiency + acute illness
  • Solucortef IV, IM
    • 0-3 years: 25 mg
    • 3-12 years: 50 mg
    • >12 years: 100 mg

• Venous sinus thrombosis- Dr. Hill-Norby
  • 89% present with headache but can also present with altered mental status, focal neuro deficits, seizures, nuchal rigidity
  • Cavernous sinus
    • Ocular signs dominate d/t cranial enerve dysfunction
    • Cortical vein occlusions can present with motor and sensory dysfunction
  • Physical exam
    • Papilledema on fundoscopic or ultrasound
      • Ultrasound measurement is measured 3 mm posterior to the retina
  • Dx
    • CT/CTV
    • MRI/MRV
    • LP with opening pressure can be suggestive of Dx
  • Tx
    • Recanalize occlusion
    • Prevent propagation
    • Treat underlying cause
    • Standard care for elevated icp (HOB elevation to 30 degrees, etc.)
    • Seizure prophylaxis
• PRES- Dr. McMurray
  • Sx usually will have posterior cortical deficits
  • 25% of people with PRES will not have HTN on presentation
  • Risk factors include renal disease, autoimmune conditions and immunosuppressive Tx
  • Pathogenesis
    • Autoregulatory failure, endothelial dysfunction, cortical dysfunction 
  • Tx
    • Target maximal reduction in MAP by 20-25% in the first hour
    • Reduce to 160/100 over next 2-6 hours
    • Then to normal over the next 24-48 hours
    • Medications
      • Labetalol, cardene, hydralazine, nitro
      • Seizure medications for seizures, if suspect eclampsia give Mg
• Emergency management of individuals with brain tumors, a focus on steroids- Dr. Mistry
  • Focus of ER management
    • Control ICP (nonsurgically)
      • locally high ICP can progress to a generalized ICP problem
        • generalized will eventually involve the brainstem, also concerning is focal ICP that can compress the brain stem
      • signs of brain stem compression
        • imaging showing posterior fossa or supratentorial lesion/hydro
        • decreased mental status
        • bradycardia
        • hypertension (especially the diastolic pressure)
      • control
        • Delay MRI until after addressing ICP
        • Position
          • HOB > 30 degrees
            • Works by increasing venous return
          • Neck in anatomically free position
            • Want the jugular veins to actually be able to return blood
        • Vital interventions
          • Intubation
          • Hyperventilate (ETCO2 ~ 25 mmHg)
        • Drugs
          • Mannitol +/- furosemide
            • Will break down the blood brain barrier and will only work once
          • Hypertonic NaCl (>3%)
            • Preferred, can be given more than once and help control ICP
    • Control of tumor-related hemorrhage (ICP)
    • Control of neuroendocrine related shock
    • Control of seizures
      • Especially vulnerable are patients with temporal lobe lesions
  • Dexamethasone- “ a big problem”
    • Evidence for dex was initially based on case series work
    • However, there is NO evidence for dexamethasone, there is not even 1 study on dex that shows benefit
    • Dexamethasone is a very potent and long acting anti-inflammatory
      • Can be bad for people needing a stem-cell transplant 
      • Kills lymphocytes by apoptosis
        • *Pre-operative dexamethasone decreases diagnostic yield from surgical samples of primary CNS lymphoma*
    • Study in Brain 2016 showed that corticosteroids decreased survival in glioblastoma 
    • Pre-op dexamethasone in 2021 Hopkins study showed greatly decreased survival on Kaplan-Meyer survival curve
    • Dexamethasone thwarts immunotherapy
      • Combined corticosteroids plus immunotherapy has a higher hazard ratio than immunotherapy alone
    • Dexamethasone is standard of care and now we are in a battle with reversing this narrative
      • **dexamethasone does not decrease ICP emergently, it can take a week to see the ICP effects, use mannitol, Lasix, or hypertonic saline**
• **there is one type of tumor to give steroids**
  • Pituitary apoplexy- a special hemorrhage
    • ER treatment is counter adrenal crisis (hydrocortisone 100 or 200 mg) and give fluids
      • Need to draw all endocrine labs before giving the hydrocortisone
    • Need a CTA immediately because there is an aneurysm that will mimic pituitary apoplexy, r/o aneurysm first before they can take to the OR
    • Consult
      • NES, ENT, optho, and endocrinology